Acromegaly

Synonyms in a broader sense

pituitary giant growth, growth disturbance English: acromegaly, pituitary gigantism

Definition Acromegaly

Acromegaly is an enlargement of the acra (see below) and the internal organs due to an increased secretion of growth hormone (somatotropin, GH (Growth Hormone)). This excessive secretion is present after the completion of longitudinal growth. Acres are for example nose, lips, tongue, ears, hands, fingers and feet. If this overproduction occurs before puberty, i.e. if the length growth is not yet completed, a giant growth (pituitary giantism) develops, caused by the pituitary gland.

History

The term acromegaly comes from the Greek akron = tip and mega = large. The first describer of this clinical picture today is the neurologist Pierre Marie from Paris. At the end of the 19th century, he recognized the enlargement of the acres (see definition) as a clinical picture.

However, there are indications that acromegaly was already known to the Egyptians at the time of the pharaohs. There are portraits of pharaohs with bulging lips, large chin and protruding nose. At that time these typical anatomical features of acromegaly were considered divine. – Cerebrum

  • Cerebellum
  • Spinal cord
  • Pituitary gland

Frequency /Epidemiology

Occurrence in the population On average, about 40 – 70 people out of 1 million inhabitants fall ill. Every year, about 3-4 people per 1 million inhabitants fall ill again.

Cause of acromegaly

Usually there is an adenoma (benign tumour) of the anterior pituitary gland (this is a hormone producing part of the brain), which produces the hormone somatotropin in excessive amounts. An adenoma is a benign tumour that originates from glandular cells and attempts to imitate them. In this case it grows in the front part of the pituitary gland.

In extremely rare cases the tumour can also be malignant (carcinoma). The following causes are equally rare: an overproduction of somatotropin, the cause of which lies outside the anterior pituitary gland. Since the production of this hormone is stimulated by the so-called GHRH (growth hormone releasing hormone) from the hypothalamus (another region of the brain), in very rare cases, tumour-induced overproduction can also occur here. This increased release would then also lead to an increased release of the hormone somatotropin (growth hormone).

Symptoms / Complaints

Typical symptoms of acromegaly are enlargement and coarsening of the facial skull, hands and feet. This enlargement of the acra is almost always present, as is thickening of the skin and splanchnomegaly (abnormal enlargement of the internal organs). In women, acromegaly often leads to menstrual disorders (disturbance of the monthly period).

In almost half of the men, libido and potency disorders occur. Both sexes experience increased sweating (hyperhidrosis). In patients with acromegaly, the protruding eye bulges (prominent supraorbital bulges) are also noticeable on the face.

The enlargement of the nose, lips and tongue leads to a typically clumsy speech. Depending on the size of the adenoma, headaches and visual disturbances are repeatedly observed due to its suppressive growth. The peripheral nervous system can also be affected: there are sensory disturbances (numbness) in the hands and feet and also muscle weakness. In 35-50% of patients a carpal tunnel syndrome with compression of the median nerve is seen.